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Bilateral sensorineural deafness in adenosine deaminase-deficient severe combined immunodeficiency
Wendy Albuquerque1, Hubert B Gaspar
1Department of Audiology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.
The Journal of Pediatrics
|February 5, 2004
Abstract:
Adenosine deaminase deficiency presents with severe combined immunodeficiency and is treatable by bone marrow transplantation. With improved survival, the nonimmunologic manifestations of this condition are becoming apparent. We report a high incidence of bilateral sensorineural deafness in transplanted patients, which highlights the systemic nature of the disease.